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Surgical experience in patients with Marfan's syndrome, ascending aortic aneurysm and aortic regurgitation
Abstract:
Surgical treatment of cardiovascular complications in patients with Marfan's syndrome is usually recommended with apprehension since the systemic nature of the disease predisposes to early and late complications. To define the incidence of these complications, 30 patients were evaluated after surgical treatment of aortic insufficiency and ascending aortic aneurysm at the Texas Heart Institute. To provide a minimal follow-up period of 5 years, only patients operated upon during of before 1968 were included in this series. There were 9 female and 21 male patients aged 4 to 80 years (mean 44 years). Aortic insufficiency was treated by valvuloplasty in 3 patients and by aortic valve replacement in 27. Graft replacement of the ascending aorta was required in 23 patients, and the aneurysm was excised and the aorta repaired by direct anastomosis in 7. Two patients were lost to follow-up study; 12 of the remaining 28 (42.8 percent) lived 5 years or more. The hospital mortality rate was 20 percent (6 of 30); the causes of death included dissection or rupture of the aorta in three patients, congestive heart failure in two and pulmonary embolism in one. The 24 survivors lived from 5 weeks to 9 years. Follow-up data were available on 22 of these patients. Ten of these (45.4 percent) died of late complications. Seven died suddenly, four of these had redissection, one patient had occlusion of the right coronary artery, and two had ventricular fibrillation of no apparent cause. The remaining three died of noncardiac causes. Although the risk of ascending aortic and aortic valve surgery in patients with Marfan's syndrome is high, 42.8 percent of the patients in our series survived 5 years or more. We believe that surgery should be recommended for patients with Marfan's disease who have dissection of the aorta or severe aortic regurgitation, or both.
Insights
Surgical treatment for Marfan syndrome cardiovascular issues carries high risks, but offers a survival benefit. Over 42% of patients in this study survived 5 years or more after aortic surgery.
Area of Science:
- Cardiovascular Surgery
- Genetics
- Internal Medicine
Background:
- Marfan syndrome is a systemic genetic disorder affecting connective tissue.
- Cardiovascular complications, particularly aortic root and valve issues, are a major cause of morbidity and mortality in Marfan syndrome.
- Surgical intervention for these complications is often approached with caution due to disease-related risks.
Purpose of the Study:
- To evaluate the incidence of early and late complications after surgical treatment of aortic insufficiency and ascending aortic aneurysm in Marfan syndrome patients.
- To determine the long-term survival rates and identify risk factors for mortality in this patient cohort.
Main Methods:
- A retrospective review of 30 patients with Marfan syndrome who underwent surgical treatment for aortic insufficiency and/or ascending aortic aneurysm.
- Surgical procedures included aortic valve replacement, valvuloplasty, graft replacement of the ascending aorta, and aortic aneurysm repair.
- Follow-up data were collected for a minimum of 5 years, with analysis of hospital mortality and late complications.
Main Results:
- The hospital mortality rate was 20% (6 of 30), with causes including aortic dissection/rupture, congestive heart failure, and pulmonary embolism.
- Of the 28 patients with follow-up, 42.8% survived 5 years or more.
- Late complications led to death in 45.4% of survivors, with causes including sudden death, aortic redissection, and ventricular fibrillation.
Conclusions:
- Despite the high risks associated with ascending aortic and aortic valve surgery in Marfan syndrome, a significant proportion of patients can achieve long-term survival.
- Surgery should be considered for Marfan syndrome patients presenting with aortic dissection or severe aortic regurgitation.
- Careful patient selection and surgical management are crucial for improving outcomes in this population.